Innovation Anthology #427:
Researchers with PrioNet and at the University of Alberta have made an exciting new discovery about ALS or Lou Gehrig’s disease.
They’ve shown that this neurodegenerative disease develops in the same manner as misfolded prion diseases like “mad cow” and Creutzfeld-Jacobs disease.
As Dr. David Wishart from the U of A expains, the culprit is a protein called super oxide dismutase.
DR DAVID WISHART: Like the prion, the super oxide dismutase or SOD1 sometimes will spontaneously misfold. When it does that, it will cause other SOD1 proteins to misfold. And when they start misfolding they start clumping. And when they start clumping, they start killing the cells that they are in. And that cell death is what leads to the pathologies of Lou Gehrig’s disease. And it seems to progress and spread from a point and move down to different parts of the brain and different parts of the motor neurons.
Researchers still don’t know what triggers SOD1 to misfold, but Dr. Wishart says they can now start working on vaccines or drugs to prevent the development of Lou Gehrig’s disease.
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I’M CHERYL CROUCHER
Guest
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Sponsor
Canadian Institutes of Health Research
Program Date: 2011-09-22